1 Preface
TJ Steiner
|
P Martelletti
|
Global Campaign Director | President |
Lifting The Burden
| European Headache Federation |
2 European principles of management of headache disorders in primary care
2.1 Introduction
2.2 Development process
2.2.1 Stakeholder involvement
2.2.2 Rigour of development
2.2.3 Editorial independence
2.3 The principles
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General aspects of headache management (Additional file 4)
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Advice to patients (Additional file 5)
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Management of tension-type headache (Additional file 10)
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Management of cluster headache (Additional file 11)
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Management of medication-overuse headache (Additional file 12)
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Management of trigeminal neuralgia and persistent idiopathic facial pain (Additional file 13)
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Headache management in primary care: when to refer (Additional file 14)
2.3.1 Clarity and presentation
2.3.2 Applicability
2.4 Guides to diagnosis
2.4.1 Headache as a presenting complaint
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▪ Primary headache disorders include migraine, tension-type headache (TTH) and cluster headache, all of which are important in primary care (Table 1).
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▪ Secondary headache disorders have another causative disorder underlying them; therefore, the headache occurs in close temporal relation to the other disorder, and/or worsens or improves in parallel with worsening or improvement of that disorder. These associations are keys to their diagnosis. Secondary headache disorders include medication-overuse headache (MOH), also important in primary care (Table 1).
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▪ Painful cranial neuropathies and other facial pains include two disorders, trigeminal neuralgia and persistent idiopathic facial pain, that need to be recognised in primary care.
Migraine | • Usually episodic, occurring in 15–25% of the general population, in women more than men in a ratio of up to 3:1; • A chronic type is recognised, with headache occurring on more days than not |
Tension-type headache | • Usually episodic, affecting most people from time to time but, in at least 10%, recurring frequently; • In up to 3% of adults and some children it is chronic, occurring on more days than not |
Cluster headache | • Extremely intense and frequently recurring but short-lasting headache attacks, affecting up to 3 in 1000 men and up to 1 in 2000 women |
Medication-overuse headache | • A secondary headache, but occurring only as a complication of a pre-existing headache disorder, usually migraine or tension-type headache, present on most days (≥15 days/month) and affecting 1–2% of adults, women more than men, and about 0.5% of children and adolescents |
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▪ The exception is chronic migraine. This uncommon type should be recognised in primary care, but it is difficult to treat and likely to require specialist management.
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▪ Specific advice on this is given below (also, Additional file 9).
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▪ Cluster headache should be diagnosed in primary care because it is easily recognisable, but referred for specialist management.
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▪ Specific advice on this is given below (also, Additional file 11).
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▪ Among painful cranial neuropathies and other facial pains are trigeminal neuralgia and persistent idiopathic facial pain. These should be recognised when present, but require specialist management.
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▪ Specific advice on each of these is also given below (also, Additional file 13).
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▪ Any headache not responding satisfactorily to management in primary care should also be referred for specialist management.
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▪ Of the large number of other secondary headache disorders, some are serious. Overall these account for <1% of patients presenting with headache, but they must be recognised.
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▪ Advice on these is provided under 2.4.3 Diagnosis of headache disorders (also, Additional file 3).
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2.4.2 Typical features of the headache disorders relevant to primary care
Migraine | Tension type headache (TTH) | Cluster headache (CH) | |
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Temporal pattern | Episodic migraine: Recurrent attack-like episodes, lasting from 4 h to 3 days; frequency often 1–2/month but variable from 1/year to 2/week or more; freedom from symptoms between attacks Chronic migraine: Episodicity lost: headache on ≥15 days/month, having migrainous features on ≥8 days/month | Frequent episodic TTH: Recurrent attack-like episodes lasting hours to a few days; 1–14 days affected per month; freedom from symptoms between attacks Chronic TTH: ≥15 days affected per month (often daily and unremitting) | Episodic CH: Frequent (typically ≥1 daily) short-lasting attacks (15–180 min): • Recurring in bouts, usually once or sometimes twice a year, which are typically of 6–12 weeks’ duration; • Then remitting for ≥3 months Chronic CH: Similar, but without such remissions between bouts |
Typical headache characteristics | Often unilateral; often pulsating | Can be unilateral but more often generalised; may spread to the neck; typically described as pressure or tightness | Strictly unilateral (although side-shifts occur occasionally), around the eye or over the temple |
Headache intensity | Typically moderate to severe | Typically mild to moderate | Extremely severe |
Associated symptoms | Aura (in a minority of attacks); often nausea and/or vomiting; often photo- and/or phonophobia | Frequent episodic TTH: None typical; mild photophobia or phonophobia may occur Chronic TTH: Sometimes mild nausea, but not vomiting | Strictly ipsilateral autonomic features: • Any or all of red and/or watering eye, running or blocked nostril, ptosis |
Reactive behaviour | Avoidance of physical activity (maybe bed rest); preference for dark and quiet | None specific | Marked agitation: cannot lie still during attacks |
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▪ recurrent episodic moderate or severe headaches which, typically but not always:
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▪ are unilateral and/or pulsating;
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▪ last (when untreated) from 4 h to 3 days;
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▪ are associated with:
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▪ nausea and/or vomiting;
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▪ photophobia, phonophobia and sometimes osmophobia;
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▪ are aggravated by routine physical activity, and disabling;
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▪ and during which they limit their activity and prefer dark and quiet;
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▪ freedom from these symptoms between attacks.
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▪ attacks may be shorter-lasting;
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▪ headache is more often bilateral and less often pulsating;
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▪ gastrointestinal disturbance is often more prominent.
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▪ aura preceding or less commonly accompanying headache and consisting of one or more neurological symptoms (see Table 3)
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▪ headache that is similar to migraine without aura, or may be rather featureless.
Typical | • Visual symptoms (occurring in >90% of auras): usually a slowly-enlarging scintillating scotoma (patients may draw a jagged crescent if asked); and/or • Unilateral paraesthesiae and/or numbness of hand, arm and/or face |
Less usual | • Brainstem symptoms (eg, vertigo, tinnitus, diplopia, ataxia); • Speech and/or language disturbances |
Rare | • Motor weakness |
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▪ headache occurring on ≥15 days/month for at least 3 months which:
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▪ on ≥8 days/month meets diagnostic criteria for migraine (or responds to migraine-specific drug treatment);
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▪ depression and/or anxiety;
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▪ low back and/or neck pain;
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▪ medication overuse.
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▪ the correct diagnosis is then medication-overuse headache (MOH);
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▪ chronic migraine and MOH are not mutually exclusive but, when medication is being overused, it may be that only MOH and not chronic migraine is present.
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▪ occurs in attack-like episodes on 1–14 days/month, each lasting hours to a few days;
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▪ can be unilateral but is more often generalised;
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▪ is typically described as pressure or tightness like a vice or tight band around the head, often spreading to the neck;
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▪ lacks the associated symptom complex of migraine.
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▪ occurs by definition on ≥15 days/month for >3 months, and may be daily and unremitting;
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▪ may be associated with mild nausea.
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▪ are characterised by headache of excruciating intensity, which is
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▪ strictly unilateral and localised around the eye or over the temple;
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▪ accompanied by highly characteristic and strictly ipsilateral autonomic features, including any or all of:
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▪ red and watering eye;
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▪ running or blocked nostril;
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▪ ptosis;
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▪ associated with marked agitation (the patient, unable to stay in bed, paces the room, even going outdoors);
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▪ occur once or more daily, very often at night (causing awakening);
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▪ last 15–180 min (commonly 30–60).
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▪ occurs in bouts (clusters) of recurring attacks, typically once or twice a year, which:
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▪ are of 6–12 weeks’ duration (but may be longer);
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▪ then remit until the next cluster, at least 3 months later.
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▪ persists, still as recurring attacks but without remissions, or with remissions of <3 months;
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▪ may develop from and/or revert to episodic cluster headache.
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▪ occurs daily or near-daily (by definition on ≥15 days/month);
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▪ is present – and often at its worst – early in the morning;
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▪ is causally associated with regular use, over >3 months, of:
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▪ non-opioid analgesics on ≥15 days/month, and/or
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▪ opioids, ergots or triptans, or any combination of these, on ≥10 days/month.
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▪ Trigeminal neuralgia (TN) affects women twice as commonly as men, and mostly those above 50 years of age (but may occur in younger people). It has no other known risk factors.
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▪ It is often associated with neurovascular compression of the trigeminal nerve close to its point of entry to the brainstem (classical trigeminal neuralgia).
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▪ TN is one of the most painful disorders, demanding accelerated specialist referral, investigation and treatment.
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▪ MRI of the brain (including brainstem) is essential.
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▪ This may demonstrate neurovascular compression, but is required in any case to exclude secondary causes that give rise to pains indistinguishable from classical TN. These occur more often in younger people.
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▪ occurs in bouts of repeated, stabbing or electric-shock-like pains in the distribution of one or more divisions of the trigeminal nerve (usually the 2nd and/or 3rd), which are:
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▪ excruciating;
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▪ of sudden onset;
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▪ highly characteristically triggered by sensory stimuli to the affected side of the face (touching, washing, applying make-up) or by talking, eating, chewing, drinking or smoking;
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▪ short-lasting (from less than a second up to 2 min);
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▪ strictly unilateral, and not switching side between bouts;
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▪ often serial, with up to hundreds of pain paroxysms during 1 day;
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▪ may also feature a constant aching pain between attacks, in the affected area, of moderate intensity.
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▪ has characteristics similar to classical trigeminal neuralgia, but is secondary to another disorder (usually cerebellopontine angle tumour, AV-malformation or multiple sclerosis).
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▪ is dull, aching or nagging;
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▪ recurs daily for >2 h and persists over >3 months;
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▪ is unassociated with neurological deficit;
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▪ is aggravated by stress.
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▪ patients are often referred for exclusion of sinus and dental problems, then returned untreated to primary care;
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▪ referral to a specialist clinic with a pain management programme is preferable.
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▪ the pain associated with TMD is usually most prominent in the pre-auricular areas of the face, masseter muscles and/or temporal regions;
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▪ there is significant overlap between TMD and tension-type headache and jaw, dental and bite disorders.
2.4.3 Diagnosis of headache disorders
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▪ Each of the primary headaches is in the differential diagnosis of each of the others.
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▪ Medication-overuse headache is in the differential diagnosis of chronic migraine or chronic tension-type headache.
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▪ The distinguishing features of these are described above (2.4.2 Typical features of the headache disorders relevant to primary care) (also in Additional file 2).
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▪ Otherwise, the differential diagnosis potentially includes a small number of serious secondary headaches that are important to recognise (see Warning features in the history or on examination, below).
How many different headaches types does the patient have? A separate history is needed for each. | |
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Time questions | • Why consulting now? • How recent in onset? • How frequent, and what temporal pattern (episodic or daily and/or unremitting)? • How long do headache episodes last? |
Character questions | • Manner and speed of headache onset (abrupt, progressive over minutes, hours, days or longer)? • Intensity of pain? • Nature and quality of pain? • Site and spread of pain? • Associated symptoms? |
Cause questions | • Predisposing and/or trigger factors? • Aggravating and/or relieving factors? • Family history of similar headache? |
Response questions | • What does the patient do during the headache? • How much is activity limited or prevented? • What medications are used, and how frequently? |
State of health between attacks | • Completely well, or residual symptoms? |
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▪ any new headache, or a significant change in headache characteristics, should provoke a new diagnostic enquiry;
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▪ very frequent headache should always lead to detailed enquiry into medication use, since overuse is a likely cause;
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▪ in addition, there are a number of specific warning features (“red flags”) that may be elicited (Table 5).
Warning feature | What to beware of |
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Thunderclap headache (intense headache with “explosive” or abrupt onset) | Subarachnoid haemorrhage |
Headache with atypical aura (duration >1 h, or including motor weakness) | TIA or stroke |
Aura without headache in the absence of a prior history of migraine with aura | TIA or stroke |
Aura occurring for the first time in a patient during use of combined hormonal contraceptives | Risk of stroke (requires discontinuation) |
New headache within 3 months of head trauma | Subdural haematoma |
Progressive headache, worsening over weeks or longer | Intracranial space-occupying lesion |
Headache aggravated by postures or manoeuvres that raise intracranial pressure | Intracranial space-occupying lesion |
Headache brought on by coughing, exercise or sexual activity | Intracranial space-occupying lesion |
Mild-to-moderate progressive or recurrent headache with irritability, dizziness (light-headedness), nausea and/or tiredness and confusion | Carbon monoxide poisoning |
Headache associated with unexplained focal neurological symptoms or with epileptic seizures | Suggests secondary headache |
Headache associated with change in memory or personality | Suggests secondary headache |
Headache associated with weight-loss | Suggests secondary headache |
New headache in a patient older than 50 years | Temporal arteritis or intracranial tumour |
New headache in a patient with a history of cancer or immunodeficiency (including HIV infection) | Likely to be secondary headache |
New headache in a patient with a history of polymyalgia rheumatica | Temporal (giant cell) arteritis |
New headache in a patient with a family history of glaucoma | Glaucoma |
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▪ Blood pressure measurement in all cases is good practice.
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▪ Physical examination is mandatory when the history is suggestive of secondary headache, and then may elicit warning signs (Table 6).
Warning feature | What to beware of |
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Otherwise unexplained pyrexia | Meningitis |
Neck stiffness | Meningitis or subarachnoid haemorrhage |
Focal neurological signs | Secondary headache |
Disorders of consciousness or memory | |
Change in personality | |
Weight-loss or poor general condition |
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▪ Routine blood tests as a screen for general health may be worthwhile in primary care.
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▪ Special investigations, including neuroimaging, are not indicated unless the history or examination suggests headache may be secondary to another condition.
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▪ cervicogenic headache (headache caused by a disorder of the cervical spine and its component bony, disc and/or soft tissue elements, usually but not invariably accompanied by neck pain);
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▪ headache attributed to arterial hypertension (chronic arterial hypertension below 180/110 mmHg does not appear to cause headache);
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▪ headache attributed to refractive error (rare in adults, although some evidence exists for it in children);
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▪ headache attributed to “sinusitis” (a misdiagnosis commonly applied to migraine);
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▪ trigeminal neuralgia (recurrent unilateral brief electric shock-like pains, abrupt in onset and termination, limited to the distribution of one or more divisions of the trigeminal nerve and triggered by innocuous stimuli);
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▪ occipital neuralgia (paroxysmal shooting or stabbing pain in the posterior part of the scalp, in the distributions of greater, lesser and/or third occipital nerves).
2.5 Guides to management
2.5.1 General aspects of headache management
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▪ Explanation is a crucial element of preventative management in patients with migraine or frequent episodic tension-type headache, who are at particular risk of escalating medication consumption.
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▪ While patients want to know the cause of their headache, this may not be possible. Both genetic and environmental factors contribute to processes that are not well understood.
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▪ Patients may need to be persuaded that tests are not helpful.
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▪ Patients with primary headache disorders may be advised that these tend to remit with advancing age.
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▪ may lead to lifestyle compromise, either in response to attacks or in a bid to avoid them (in this way, episodic headache can have continuous impact);
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▪ has impact not only on the person with it but also on other people (family, work colleagues and employer).
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▪ Correctly identified triggers offer the possibility of avoidance (perhaps by life-style change) as a sometimes major contribution to management.
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▪ When triggers are relevant to individual patients, they are usually self-evident.
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▪ Triggers may be less readily identified when they are cumulative in their effect, jointly lowering the threshold above which attacks are initiated.
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▪ Even when they are correctly identified, triggers are not always avoidable.
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▪ The use of outcome measures is recommended to evaluate treatment and guide follow-up. The following are included here, among the management aids:
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▪ the HURT questionnaire, developed by Lifting The Burden expressly to guide management in primary care (see 3.5.2 The Headache Under-Response to Treatment (HURT) questionnaire (also, Additional file 20));
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▪ the HALT-30 Index, to record lost productive time in the preceding month (see 3.4.2 The Headache-Attributed Lost Time (HALT) Indices (also, Additional file 19));
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▪ a headache calendar (see below).
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▪ Persistent management failure is an indication for specialist referral.
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▪ Diaries, used particularly as an aid to diagnosis, are useful for:
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▪ recording symptoms and temporal patterns that contribute to correct diagnosis;
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▪ recording acute medication use or overuse prior to diagnosis;
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▪ reporting lost productive time as part of pre-treatment assessment.
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▪ Calendars, used in follow-up, are recommended in primary care for:
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▪ revealing associations with the menstrual cycle and possibly other triggers;
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▪ monitoring acute medication use or overuse during follow-up;
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▪ encouraging adherence to prophylactic medication;
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▪ recording treatment effect on headache frequency, and charting outcomes.
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2.5.2 Advice to patients
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▪ A fifth offers information on female hormones and headache (Additional file 25).
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▪ Diets. While healthy eating is always advisable, there is no reliable evidence that gluten-free, lactose-free, ketogenic or other specific diets prevent or improve headache disorders.
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▪ Biofeedback and relaxation therapies can be helpful, and are potentially useful options when drug treatments must be avoided.
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▪ Cognitive behavioural therapy may help patients develop coping strategies and better manage their symptoms. There is no good evidence to confirm benefit.
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▪ Physiotherapy has proven benefits in some patients with tension-type headache. It requires skilled and individualised therapy, which is not widely available in many countries.
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▪ Aerobic exercise. Limited data support the benefits of aerobic exercise on migraine and tension-type headache. Exercise has other important health benefits: improving physical strength, fitness and sleep, relieving depression and reducing blood pressure, cholesterol and weight.
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▪ Acupuncture has differing forms, and is highly dependent on the skill of the therapist. There is limited evidence that acupuncture can be effective in reducing intensity and frequency of migraine attacks, but large clinical trials have failed to distinguish between acupuncture and sham procedures.
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▪ Devices. Many are on the market, some very costly and promoted with insupportable claims of efficacy. “Testimonials” can be attributed to placebo effect and should be disregarded. The only clear recommendation possible is that successful trial usage should precede any expensive purchase.
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▪ A range of transcutaneous electrical nerve stimulators (TENS) and noninvasive neuromodulating devices for peripheral vagal nerve stimulation, supraorbital nerve stimulation and single-pulse transcranial magnetic stimulation are available, with evidence of efficacy in some people.
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▪ Herbals are not recommended. Clinical trials data are limited and provide no evidence of safety in prolonged use. Herbals may interfere with other medications.
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▪ Feverfew preparations on sale everywhere are highly variable in content and their toxicity is not well understood.
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▪ Butterbur has some efficacy in migraine, but preparations on sale are variable in content and not all are free of liver toxins.
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▪ Nutraceuticals are mostly not recommended. The following have some evidence for efficacy in migraine, and may be tried where preparations of pharmaceutical quality are available:
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▪ coenzyme Q10 (CoQ10) (100 mg three times daily);
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▪ magnesium (as citrate, starting at 100 mg three times daily to avoid diarrhoea, and increasing to 200 mg three times daily);
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▪ riboflavin (200 mg twice daily).
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▪ Homoeopathy is of unproven value. There is no arguable case for over-the-counter sales of homoeopathic remedies.
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▪ Reflexology has no scientific basis.
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▪ Cold packs or menthol gel applied to the head and/or neck are found by some people to relieve pain or discomfort while being harmless and inexpensive.
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▪ Dental treatment, including splints and bite-raising appliances, is of unproven value in treating headache and should be discouraged for this purpose.
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▪ Spectacles should be professionally prescribed and worn when needed, but refractive errors are rarely a cause of troublesome headache.▪ For the same reason, accommodation training, sometimes offered by optometrists, is not an accepted treatment for headache or likely to be beneficial.
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▪ Surgical procedures. No surgical procedures produce benefit in migraine or tension-type headache. Hysterectomy has no place in migraine management.
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▪ Migraine with aura and the ethinylestradiol component of combined hormonal contraceptives (CHCs) are independent risk factors for stroke in young women.
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▪ Every woman seeking hormonal contraception in primary care should be screened for migraine with aura and, if positive, offered progestogen-only contraception or non-hormonal alternatives.
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▪ Otherwise, headache is often a side-effect of CHCs (pills, patches or vaginal rings), and many women report onset or aggravation of migraine after starting them.
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▪ Such symptoms usually resolve with continued use; if not, alternatives to CHCs should be offered.
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▪ Other women, particularly those with menstrually-related migraine (without aura), report improvement, especially when CHCs are taken continuously without a week’s break.
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▪ CHCs increase risk of stroke in young women with migraine with aura, who should therefore use alternatives;
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▪ a change from migraine without aura to migraine with aura after starting CHCs is a clear signal to stop immediately;
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▪ progestogen-only contraception is acceptable with any type or subtype of migraine.
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▪ HRT is not contraindicated in migraine with or without aura;
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▪ decisions about commencing or continuing HRT should be made according to generally applicable criteria.
2.5.3 Management of migraine
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▪ Good treatment of migraine begins with education of patients, explaining their disorder and the purpose and means of management.
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▪ Impact of migraine should be assessed prior to planning treatment:
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▪ the HALT-90 Index, assessing burden in terms of lost productive time, is included here, among the management aids (see 3.4.2 The Headache-Attributed Lost Time (HALT) Indices (also, Additional file 18)).
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▪ Triggers and predisposing factors should not be overemphasised but should nonetheless be considered early in management (with life-style modification when called for).
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▪ Almost all patients with migraine will require drug therapy for acute attacks, but not necessarily prescription drugs (see 2.5.4 Acute or symptomatic management of episodic migraine (also, Additional file 7)).
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▪ Any patient who is not well controlled with acute therapy alone and whose quality of life is impaired by migraine, whether adult or child, should be offered prophylaxis in addition (see 2.5.5 Prophylactic management of episodic migraine (also, Additional file 8)).
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▪ Every patient to whom treatment is offered, or whose treatment is changed, requires follow-up to ensure that optimum treatment has been established.
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▪ migraine is a common disorder which, while it may be disabling, is benign;
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▪ it is often familial, and probably genetically inherited;
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▪ it cannot be cured but can be successfully treated;
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▪ trigger or predisposing factors are common in migraine, and should be identified and avoided or modified when possible, but not all can be;
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▪ a headache calendar helps good management by recording over time:
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▪ the symptoms and pattern of attacks (eg, menstrual relationship);
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▪ medication use (thus identifying overuse);
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▪ regular activity (eg, sport or exercise 2–3 times per week) may reduce intensity and frequency of migraine attacks.
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▪ migraine with aura and the ethinylestradiol component of CHCs are independent risk factors for stroke in women, especially in those under 50 years;
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▪ alternatives to CHCs are therefore very strongly recommended for women with migraine with aura;
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▪ a change from migraine without aura to migraine with aura after starting CHCs is a clear signal to stop immediately;
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▪ progestogen-only contraception is acceptable with any type or subtype of migraine.
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▪ HRT is not contraindicated in migraine with or without aura;
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▪ decisions about commencing or continuing HRT should be made according to generally applicable criteria.
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▪ Use of a calendar is recommended to encourage adherence with prophylactic medication and record treatment effect. An example of a simple calendar is included here among the management aids (see 3.3 Headache diary and calendar to aid diagnosis and follow-up in primary care (also, Additional file 17)).
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▪ The use of outcome measures is recommended to guide follow-up. The following are included here among the management aids:
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▪ the HURT questionnaire was developed expressly for primary care (see 3.5.2 The Headache Under-Response to Treatment (HURT) questionnaire (also, Additional file 20));
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▪ the HALT-30 Index records lost productive time during the preceding month (see 3.4.2 The Headache-Attributed Lost Time (HALT) Indices (also, Additional file 19)).
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▪ Persistent management failure is an indication for specialist referral.
2.5.4 Acute or symptomatic management of episodic migraine
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▪ All adults with episodic migraine should have access to acute medication.
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▪ Children with short-lasting attacks may respond well to bed-rest without medical treatment.
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▪ In adults and children, regular use of acute medication at high frequency (on >2 days/week) risks the development of medication-overuse headache.
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▪ Many patients seek help in identifying triggers (see below). The importance of trigger factors in migraine is nonetheless often overemphasised.
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▪ Correctly identified triggers offer the possibility of avoidance (perhaps by life-style change) as a sometimes major contribution to management.
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▪ When triggers are relevant to individual patients, they are usually self-evident.
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▪ Cyclical hormonal fluctuations may be an obvious factor in menstruating women.
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▪ Irregular lifestyle, poor sleep pattern and “stress” are important predisposing factors in anybody with migraine. Missing meals is a potent trigger factor.
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▪ Triggers may be less readily identified when they are cumulative in their effect, jointly lowering the threshold above which attacks are initiated.
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▪ Even when they are correctly identified, triggers are not always avoidable.
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▪ Contrary to popular belief, there is no “migraine diet”. The only dietary triggers with good evidential support are certain alcoholic drinks (especially red wine).
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▪ non-opioid analgesic
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▪ plus, when needed, an antiemetic.
Analgesics | Antiemetics |
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Adults | |
Non-steroidal anti-inflammatory drugs: • Acetylsalicylic acid 900–1000 mg or • Ibuprofen 400–800 mg or • Diclofenac 50–100 mg | • Domperidone 10 mg (supportive evidence of efficacy is for 20 mg, but the European Medicines Agency recommends restriction to 10 mg orally [up to three times daily] or 30 mg by suppository [up to twice daily]), or • Metoclopramide 10 mg (the European Medicines Agency restricts dosing to 10 mg [up to three times daily]) |
Or (where these are contraindicated): • Paracetamol 1000 mga | |
Or (possibly benefiting from the different mechanisms of action): • Combinations of paracetamol with acetylsalicylic acid or ibuprofen | |
Children (when needed) | |
Ibuprofen 200–400 mg according to age and weight | • Domperidone (dosage according to age and weight) |
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▪ Opioids (including codeine and dihydrocodeine) are ineffective for migraine, associated with multiple adverse effects, potentially addictive and commonly implicated in medication-overuse headache;
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▪ Barbiturates have no place in the treatment of migraine.
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▪ Use soluble analgesics (or mouth-dispersible formulations with water) when available.
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▪ Take early in the attack.
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▪ Use adequate dosage (see Table 7: in most cases, adequate doses require more than a single tablet).
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▪ A prokinetic antiemetic counters gastric stasis, an early feature of migraine, which impairs bioavailability of oral medication.
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▪ Rectal formulations (where available) may be preferable in the presence of vomiting.
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▪ Proceed to step two after three attacks without success (local guidelines may recommend trying more than one analgesic in step one before proceeding to step two).
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▪ Where available, and unless contraindicated, specific therapy (Table 8) should be offered to all patients failing step one.
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▪ Availability of drugs varies from country to country.
Almotriptan | • Tablets 12.5 mg |
Eletriptan | • Tablets 20 and 40 mg • Tablets 80 mg (not widely available) (for some people, 80 mg is effective when 40 mg is not) |
Frovatriptan | • Tablets 2.5 mg |
Naratriptan | • Tablets 2.5 mg |
Rizatriptan | • Tablets and mouth-dispersible wafers 10 mg • Tablets 5 mg (to be used when propranolol is being taken concomitantly) |
Sumatriptan | • Tablets and rapidly dissolving tablets 50 and 100 mg • Nasal spray 10 mg (licensed for adolescents) and 20 mg • Subcutaneous injection 6 mg |
Zolmitriptan | • Tablets and mouth-dispersible tablets 2.5 and 5 mg • Nasal spray 5 mg |
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▪ Ergotamine is a poor substitute for triptans: it has very low and unpredictable bioavailability, which impairs its efficacy, and poor tolerability. It is no longer recommended for routine use.
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▪ Triptans are more effective when taken while headache is still mild (but not during aura) (this instruction should be given only to patients who can reliably distinguish migraine from tension-type headache).
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▪ The initial dose of all oral triptans (except eletriptan in some cases) is one tablet.
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▪ A second dose for non-response is not recommended by most triptan manufacturers but, taken not less than 2 h after the first, may nonetheless be effective in some cases.
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▪ Triptans should not be used regularly on ≥10 days/month to avoid the risk of medication-overuse headache.
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▪ Triptans differ slightly, but there are large and unpredictable individual variations in responses to them:
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▪ one may work where another has not;
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▪ patients are best served if they can try several, in different formulations, and choose between them.
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▪ When nausea is present, domperidone 10 mg may be added.
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▪ When vomiting is present, zolmitriptan nasal spray (absorbed through the nasal mucosa) or sumatriptan subcutaneous injection may be preferred.
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▪ Efficacy of sumatriptan may be increased by combination with naproxen 500–1000 mg (there are no data on combinations of other triptans and NSAIDs).
-
▪ When all other triptans are ineffective, sumatriptan by subcutaneous injection 6 mg should be considered.
-
▪ Triptans are associated with return of symptoms within 48 h (relapse) in up to 40% of patients who have initially responded (see below).
-
▪ A repeat dose of a triptan is usually effective.
-
▪ A further relapse may occur:
-
▪ in a minority of patients, this happens repeatedly, a major management problem with high risk of developing medication-overuse headache;
-
▪ a different triptan should be tried in future attacks;
-
▪ concomitant use of a triptan and naproxen may reduce susceptibility to relapse.
-
-
▪ Triptans should not be taken during aura of migraine with aura, but at the onset of headache.
-
▪ All triptans should be avoided by people with:
-
▪ uncontrolled hypertension (one reason for measuring blood pressure);
-
▪ coronary heart disease, cerebrovascular disease or peripheral vascular disease;
-
▪ multiple risk factors for coronary or cerebrovascular disease;
-
-
▪ In the elderly, all of these are more common, and triptans should therefore be used with greater caution.
-
▪ In pregnancy: limited safety data are available only for sumatriptan, which should be used with caution and only under specialist supervision.
-
▪ In addition, there are specific precautions attached to some triptans (see pharmacopoeia).
-
▪ Failure of step one in children is an indication for specialist referral.
-
▪ No specific anti-migraine drug has been shown to have efficacy in children (under 12 years old).
-
-
▪ For adolescents (12–17 years), the following have efficacy and are approved:
-
▪ sumatriptan nasal spray 10 mg;
-
▪ zolmitriptan nasal spray 2.5 mg and/or 5 mg (in some countries).
-
-
▪ Use of a calendar is recommended to monitor acute medication use or overuse. An example of a simple calendar is included here among the management aids (see 3.3 Headache diary and calendar to aid diagnosis and follow-up in primary care (also, Additional file 17)).
-
▪ The use of outcome measures is recommended to guide follow-up. The following are included here among the management aids:
-
▪ the HURT questionnaire was developed expressly for primary care (see 3.5.2 The Headache Under-Response to Treatment (HURT) questionnaire (also, Additional file 20));
-
▪ the HALT-30 Index records lost productive time during the preceding month (see 3.4.2 The Headache-Attributed Lost Time (HALT) Indices (also, Additional file 19)).
-
-
▪ Failure of acute therapy may be an indication for prophylaxis (see below).
2.5.5 Prophylactic management of episodic migraine
-
▪ attacks cause disability on two or more days per month, and
-
▪ acute therapy has been optimised but does not prevent this, or is poorly tolerated, or
-
▪ there is a risk of over-frequent use of acute therapy, even when it is effective; and
-
▪ the patient is willing to take daily medication.
-
▪ A calendar should be kept by every patient on prophylaxis to assess efficacy and promote adherence. An example of a simple calendar is included here among the management aids (see 3.3 Headache diary and calendar to aid diagnosis and follow-up in primary care (also, Additional file 17)).
-
▪ Poor adherence is a major factor impairing efficacy of migraine prophylactics; once-daily dosing is associated with better adherence.
-
▪ The dose of any drug should start low in the suggested range and be increased in the absence of troublesome side-effects.
-
▪ Drugs that appear ineffective should not be discontinued too soon; 2–3 months may be the minimum to achieve and observe efficacy.
-
▪ Failure of one drug does not predict failure of others in a different class.
-
▪ Tapered withdrawal may be considered after 6 months of good control, and should be considered no later than after 1 year.
-
▪ Children requiring prophylactic medication should be referred for specialist assessment.
-
▪ Availability and regulatory approval vary from country to country, and many are not specifically licensed for migraine prophylaxis. Use of drugs off-licence rests on individual clinical responsibility.
-
▪ Across the range, expected benefit is no greater than 50% fewer attacks in 50% of users after 3 months of treatment (with individual benefit varying between zero and [rarely] 100%).
-
▪ Once daily dosing (as opposed to more frequent) is associated with better adherence, an important determinant of efficacy.
Beta-adrenergic blockers without partial agonism: • atenolol 25-100 mg twice daily • bisoprolol 5-10 mg once daily • metoprolol 50-100 mg twice daily or modified-release 200 mg once daily • propranolol LA 80-160 mg once to twice daily | • observe general contraindications, including comorbid depression • propranolol has best evidence of efficacy, but not evidence of best efficacy • cardioselective and non-lipophyllic drugs (bisoprolol, atenolol, metoprolol) are likely to be better tolerated |
Amitriptyline 10-100 mg at night | • may be preferred when migraine coexists with tension-type headache, depression or sleep disturbance |
Topiramate 50 mg twice daily | • titrate over 4 weeks from 25 mg once daily • contraindicated in pregnancy |
Candesartan 16 mg once daily | • start at 8 mg once daily and titrate weekly • contraindicated in pregnancy |
Sodium valproate 600-1500 mg daily | • titrate upwards • avoid altogether in women of child-bearing potential (even on contraception); absolutely contraindicated in pregnancy |
Flunarizine 5-10 mg once daily | • observe general contraindications, including comorbid depression |
CGRP monoclonal antibodies (to the peptide or its receptor): • erenumab 70 or 140 mg s/c once monthly • fremanezumab 225 mg s/c once monthly or 675 mg s/c once quarterly • galcanezumab 240 mg s/c, then 120 mg s/c once monthly | • newly licensed, not yet universally available or reimbursed, usually restricted to specialist care and reserved for those failing (or not tolerating) other prophylactics • all self-administered by auto-injector • high relative cost |
-
▪ Onabotulinum toxin A (Botox). This is not effective in episodic migraine and is not recommended for this condition.
-
▪ Surgical procedures. There is no evidence to support any surgical procedure as a treatment for episodic migraine.
-
▪ In particular, migraine is not improved by closure of patent foramen ovale (PFO). This procedure should not be undertaken for migraine prophylaxis: it carries a small but relevant risk of serious adverse events including stroke, pericardial tamponade, atrial fibrillation and death.
-
-
▪ Acupuncture has differing forms, and is highly dependent on the skill of the therapist. There is limited evidence that acupuncture can be effective in reducing intensity and frequency of migraine attacks, but large clinical trials have failed to distinguish between acupuncture and sham procedures. Benefits experienced by some patients may be attributable to placebo effect.
-
▪ Devices. Many are on the market, some very costly and promoted with insupportable claims of efficacy. “Testimonials” can be attributed to placebo effect and should be disregarded. The only clear recommendation possible is that successful trial usage should precede any expensive purchase.
-
▪ A range of transcutaneous electrical nerve stimulators (TENS) and noninvasive neuromodulating devices for peripheral vagal nerve, supraorbital nerve and single-pulse transcranial magnetic stimulation are available, with evidence of efficacy in some people.
-
-
▪ Herbals are not recommended. Evidence of both efficacy and safety in prolonged use is poor. They may interfere with other medications.
-
▪ Feverfew preparations are highly variable in content, and not all of pharmaceutical quality. Their toxicity is not well understood.
-
▪ Butterbur has some efficacy and is approved for use in some countries, but preparations on sale are variable in content and not all of pharmaceutical quality (not guaranteed to be free of liver toxins).
-
-
▪ Nutraceuticals are mostly not recommended. The following have some evidence for efficacy, and may be tried where preparations of pharmaceutical quality are available:
-
▪ coenzyme Q10 (CoQ10) (100 mg three times daily);
-
▪ magnesium (as citrate, starting at 100 mg three times daily to avoid diarrhoea, and increasing to 200 mg three times daily);
-
▪ riboflavin (200 mg twice daily).
-
-
▪ Homoeopathy is of unproven value. There is no arguable case for over-the-counter sales of homoeopathic remedies.
-
▪ This is better avoided, and rarely required since migraine often remits during pregnancy.
-
▪ Sodium valproate is absolutely contraindicated; topiramate and candesartan are contraindicated.
-
▪ Propranolol and amitriptyline have best evidence of safety, but specialist guidance is recommended.
-
▪ Riboflavin (vitamin B2), 200 mg twice daily, may be tried, but may not show efficacy for 3 months.
-
▪ Use of a calendar is recommended to encourage adherence with prophylactic medication and record treatment effect. An example of a simple calendar is included here among the management aids (see 3.3 Headache diary and calendar to aid diagnosis and follow-up in primary care (also, Additional file 17)).
-
▪ The use of outcome measures is recommended to guide follow-up. The following are included here among the management aids:
-
▪ the HURT questionnaire was developed expressly for primary care (see 3.5.2 The Headache Under-Response to Treatment (HURT) questionnaire (also, Additional file 20));
-
▪ the HALT-30 Index records lost productive time during the preceding month (see 3.4.2 The Headache-Attributed Lost Time (HALT) Indices (also, Additional file 19))
-
-
▪ Failure may be due to subtherapeutic dosage (itself perhaps due to non-adherence) or insufficient duration of treatment.
-
▪ The following actions are recommended:
-
▪ review the diagnosis;
-
▪ review adherence;
-
▪ review other medication, especially for overuse.
-
-
▪ When prophylaxis still fails to have clear benefit, discontinue it.
-
▪ When all options fail, specialist referral is indicated.
2.5.6 Management of chronic migraine
-
▪ suspected in any patient:
-
▪ with a history of migraine
-
▪ who reports (or records in a diary) headache on ≥15 days/month;
-
-
▪ diagnosed, in the absence of medication overuse, in patients with:
-
▪ headache on ≥15 days/month over the last 3 months, which
-
▪ on ≥8 days/month:
-
▪ fulfilled the diagnostic criteria for migraine, or
-
▪ responded to migraine-specific drug treatment.
-
-
-
▪ medication-overuse headache (MOH) is another syndrome characterised by headache on ≥15 days/month;
-
▪ chronic migraine and MOH are not mutually exclusive but, even when the conditions above are met, only MOH and not chronic migraine may be present when medication is being overused;
-
▪ medication overuse, whether or not occurring with chronic migraine, must always be recognised and managed as a separate medical problem.
-
▪ education of patients about chronicity and its causes and risk factors;
-
▪ recognition and management of medication overuse, when present;
-
▪ management of any comorbidities;
-
▪ use of preventative drugs (Table 10);
-
▪ follow up, with both medical and psychological care.
Topiramate, 50 mg or more twice daily | |
Onabotulinum toxin A, 155-195 units by multisite injection | • not licensed for chronic migraine in some countries, or • not reimbursed, and/or • regulators require prior failure of two or more of the drugs used in prophylaxis of episodic migraine |
CGRP monoclonal antibodies (to the peptide or its receptor): • erenumab 70 or 140 mg s/c once monthly • fremanezumab 225 mg s/c once monthly or 675 mg s/c once quarterly • galcanezumab 240 mg s/c, then 120 mg s/c once monthly | • newly licensed, not yet universally available or reimbursed, usually restricted to specialist care and reserved for those failing (or not tolerating) other prophylactics • all self-administered by auto-injector • high relative cost |
2.5.7 Management of tension-type headache (TTH)
-
▪ frequent episodic TTH, with headache attacks on 1–14 days/month on average;
-
▪ chronic TTH, one of the syndromes characterised by headache occurring on ≥15 days/month, either with highly-frequent attacks or, occasionally, continuous and unremitting.
-
▪ Good treatment of patients with troublesome TTH (of either type) begins with their education, explaining their disorder and the purpose and means of management.
-
▪ Impact of TTH should be assessed prior to planning treatment:
-
▪ the HALT-90 Index, assessing burden in terms of lost productive time, is included here, among the management aids (see 3.4.2 The Headache-Attributed Lost Time (HALT) Indices (also, Additional file 18)).
-
-
▪ Infrequent headaches (on ≤2 days/week) are managed with over-the counter (OTC) analgesics.
-
▪ When headache is more frequent:
-
▪ advice on lifestyle may be helpful, possibly accompanied by psychological intervention such as cognitive behavioural therapy;
-
▪ analgesics (even OTC) should be used with care because of the risk of medication-overuse headache;
-
▪ prophylaxis may be indicated.
-
-
▪ TTH is a very common disorder but, while it may be disabling and troublesome when headaches are frequent, it is benign;
-
▪ episodic TTH can be successfully treated, usually with OTC analgesics;
-
▪ over-frequent use of medications, even OTC, will make headaches worse;
-
▪ chronic TTH cannot be regularly treated with analgesics and usually requires other long-term continuous medication and/or non-pharmacological interventions;
-
▪ a headache calendar helps good management by recording over time the symptoms and pattern of attacks and medication use;
-
▪ predisposing factors sometimes include stress and/or poor head and neck posture;
-
▪ regular activity (eg, sport or exercise 2–3 times per week) may help frequent TTH.
Ibuprofen 400–800 mg | • For adults, and • Drug of choice for children (200–400 mg according to age and weight) |
Acetylsalicylic acid 600–1000 mg | • Adults only |
Either of these in combination with paracetamol 1000 mg | • Formal evidence is lacking, but the different mechanisms of action may enhance effect |
Any of these in combination with caffeine | • Commonly included in analgesic combination-medications |
Paracetamol 1000 mg | • On its own has lower efficacy • Therefore reserved for those in whom NSAIDS are contraindicated |
-
▪ Opioids (including codeine and dihydrocodeine) are ineffective for headache, associated with multiple adverse effects, potentially addictive and commonly implicated in medication-overuse headache.
-
▪ Barbiturates have no place in the treatment of TTH.
-
▪ Metamizol has limited evidence for efficacy and is associated with agranulocytosis.
-
▪ Triptans are specific for migraine, and ineffective in TTH.
-
▪ Episodic TTH occurring on ≤2 days/week can usually be successfully treated with OTC analgesics alone;
-
▪ As the frequency of headaches increases, so does the risk of medication overuse:
-
▪ episodic TTH on >2 days/week is a clear indication for prophylaxis (see below) in place of, rather than in addition to, acute intervention;
-
▪ acute treatments are unlikely to be effective in chronic TTH and put the patient at clear risk of medication-overuse headache.
-
-
▪ A calendar should be kept to assess efficacy and promote adherence. An example of a simple calendar is included here among the management aids (see 3.3 Headache diary and calendar to aid diagnosis and follow-up in primary care (also, Additional file 17)).
-
▪ Patients receiving medication more often used as an antidepressant should be advised of this, and why; otherwise, they may default when they find out.
-
▪ Prophylaxis that appears ineffective should not be discontinued too soon; 2–3 months may be the minimum to achieve and observe efficacy.
-
▪ Tapered withdrawal may be considered after 6 months of good control, but prolonged treatment is sometimes indicated.
Amitriptyline, 10–100 mg at night | • Drug of choice for frequent episodic or chronic TTH; • Intolerance is reduced by starting at a low dose (10 mg) and incrementing by 10–25 mg each 1–2 weeks |
Nortriptyline (replacing amitriptyline at the same dose) | • Fewer anticholinergic side-effects but less good evidence of efficacy |
Mirtazapine, 15–30 mg once daily | • Second-line option |
Venlafaxine, 75–150 mg once daily | • Third-line option |
-
▪ Onabotulinum toxin A is ineffective in TTH.
-
▪ There is limited evidence that acupuncture is effective in reducing intensity and frequency of TTH episodes. While some patients experience benefit, this may be due to placebo effect. Acupuncture has differing forms, and is highly dependent on the skill of the therapist.
-
▪ There is well-documented evidence of efficacy of various forms of biofeedback. They are highly dependent on the skill of the therapist.
-
▪ Use of a calendar is recommended to monitor acute medication use or overuse, or to encourage adherence to prophylactic medication, and to record treatment effect. An example of a simple calendar is included here among the management aids (see 3.3 Headache diary and calendar to aid diagnosis and follow-up in primary care (also, Additional file 17)).
-
▪ The use of outcome measures is recommended to guide follow-up. The following are included here among the management aids:
-
▪ the HURT questionnaire was developed expressly for primary care (see 3.5.2 The Headache Under-Response to Treatment (HURT) questionnaire (also, Additional file 20));
-
▪ the HALT-30 Index records lost productive time during the preceding month (see 3.4.2 The Headache-Attributed Lost Time (HALT) Indices (also, Additional file 19)).
-
-
▪ Failure may be due to subtherapeutic dosage (itself perhaps due to non-adherence) or insufficient duration of treatment.
-
▪ The following actions are recommended:
-
▪ review the diagnosis;
-
▪ review adherence;
-
▪ review other medication, especially for overuse;
-
-
▪ When prophylaxis still fails to have clear benefit, discontinue it.
-
▪ When all options fail, specialist referral is indicated.
-
▪ Despite best efforts, chronic TTH is often refractory to medical treatment or may become so.
-
▪ Patients in this situation require referral into a pain management programme with emphasis on psychological approaches.
2.5.8 Management of cluster headache
-
▪ Cluster headache is easily recognisable (see 2.4.2 Typical features of the headache disorders relevant to primary care (also, Additional file 2)).
-
▪ It should never be missed.
-
▪ episodic cluster headache, with attacks occurring in bouts (clusters) that last for a few or many weeks and then remit for ≥3 months;
-
▪ chronic cluster headache, less common, but persisting without remissions, or with remissions of <3 months.
-
▪ Patients with this disorder suffer very badly if ineffectively treated:
-
▪ cluster headache management is, at least initially, better left to specialists who see this disorder frequently;
-
▪ on first presentation it demands accelerated referral for investigation and treatment;
-
▪ recognition in primary care is crucial to ensure prompt referral.
-
-
▪ The objective of management in both episodic and chronic subtypes is total attack suppression. This is not always achievable.
-
▪ Both acute medication and prophylaxis have a role in management, but preventative drugs are the mainstay of treatment in most cases.
-
▪ Once effective treatment has been established, future clusters, or maintenance therapy in the case of chronic cluster headache, may be managed in primary care.
-
▪ Availability varies between countries.
-
▪ Most are not specifically licensed for cluster headache. Use of drugs off-licence rests on individual clinical responsibility.
Triptans: | None can be recommended for use more than twice a day |
• Sumatriptan 6 mg s/c | • The most highly-effective acute treatment |
• Zolmitriptan 5 mg nasal spray | • Less-certain efficacy but an alternative for those unable or unwilling to use sumatriptan s/c |
• Sumatriptan 20 mg nasal spray | • Less-certain efficacy: absorption depends largely on ingestion |
Oxygen 100% at ≥12 l/min until response, or for ≥15 min | • Requires a non-rebreathing mask and regulator; • Helps some people and may be used as frequently as needed |
-
▪ Oral triptans are slow in onset of action and are not useful substitutes.
-
▪ Analgesics, including opioids, have little or no place in treating cluster headache.
Prednisolone 60–80 mg once daily | • For 2–4 days, discontinued by dose reduction over 1–3 weeks |
Greater occipital nerve blockade | • Using various agents |
Verapamil 240–960 mg daily | • ECG monitoring advised |
Lithium carbonate 600–1600 mg daily | • Serum levels must be regularly monitored |
Topiramate 50–100 mg twice daily | • Less evidence of efficacy, but no monitoring required |
-
▪ Prophylaxis of episodic cluster headache should begin as early as possible after the start of a new cluster bout.
-
▪ Failure of one drug does not predict failure of others.
-
▪ Combinations of drugs may be tried, but the potential for toxicity is obviously high.
-
▪ For episodic cluster headache, maintenance prophylaxis should be discontinued by tapering, usually 2 weeks after full remission.
-
▪ For chronic cluster headache, maintenance prophylaxis may need to be continued long-term.
-
▪ Neuromodulation, non-invasive or invasive, is occasionally used by specialists.
-
▪ Patients with episodic cluster headache in remission should be advised to return promptly at the onset of the next cluster episode.
2.5.9 Management of medication-overuse headache (MOH)
-
▪ Prevention, through education, is preferable to cure.
-
▪ Once MOH has developed, early intervention has better chance of success.
-
▪ The necessary management of established MOH is to stop overuse of the suspected medication(s).
-
▪ Patient education, that medication taken to relieve headache is in fact its cause, is the essential first step:
-
▪ success in management depends crucially on patients’ understanding that their medication taken to relieve their headache is in fact its cause.
-
-
▪ Management is usually possible in primary care.
-
▪ The long-term prognosis is usually very good. Most cases revert to episodic headache, although the outcome depends on:
-
▪ the type of headache from which MOH developed;
-
▪ the class of medication overused (opioids causing greatest difficulty);
-
▪ the duration of overuse;
-
▪ comorbidities (psychiatric, or other causes of chronic pain).
-
-
▪ The “treatment” a patient is taking for headache is actually the cause of it.
-
▪ Effective treatment requires, in the first instance, stopping use of the suspected medication(s) (withdrawal):
-
▪ there is no other option;
-
▪ many patients recover from this alone.
-
-
▪ Initial worsening of symptoms for 1–2 weeks during and after withdrawal must be expected.
-
▪ The outcome is usually very good, with reversion in most cases, within 2 months, to the antecedent episodic headache disorder.
-
▪ stop the overused medication;
-
▪ recovery from MOH (which should follow);
-
▪ review and reassess the underlying headache disorder (usually migraine or tension-type headache);
-
▪ prevent relapse, while allowing acceptable use of medications.
-
▪ Worsening headache for 1–2 weeks is almost inevitable:
-
▪ accordingly, withdrawal should be planned to avoid unnecessary lifestyle disruption;
-
▪ 1–2 weeks’ sick leave may be needed;
-
▪ admission to hospital during withdrawal is rarely necessary unless:
-
▪ overused medication(s) include opioids;
-
▪ for management of comorbidities.
-
-
-
▪ Withdrawal may be undertaken in any of three ways, the choice being made by the patient:
-
▪ abruptly:
-
▪ there is evidence that this is the most successful approach;
-
-
▪ by tapering over a period of 2–4 weeks:
-
▪ withdrawal symptoms are likely to be less intense but more prolonged;
-
-
▪ by replacing the overused medication(s) with naproxen 500 mg twice daily for 3–4 weeks and no longer:
-
▪ the purpose is to break the behavioural “have headache – take medication” link;
-
▪ many patients become headache-free on this medication;
-
▪ naproxen must be stopped after this period (never continued).
-
-
-
▪ Headache usually shows signs of improvement 1–2 weeks after stopping overused medication(s).
-
▪ Recovery continues slowly for up to 2 months.
-
▪ Prophylaxis against the antecedent headache (most often migraine) may be introduced on its return, or commenced in parallel with the withdrawal process.
-
▪ First review is advised after 2–3 weeks to ensure withdrawal has been successfully achieved.
-
▪ Use of a calendar during withdrawal is strongly recommended to record symptoms and medication use, and to record changing headache pattern. An example of a simple calendar is included here among the management aids (see 3.3 Headache diary and calendar to aid diagnosis and follow-up in primary care (also, Additional file 17)).
-
▪ Most patients revert to their antecedent headache (usually migraine or tension-type headache) within 2 months; this will need review and appropriate management.
-
▪ The relapse rate is high within the first year: further follow-up is important to avoid it, and many patients require extended support.
-
▪ Previously overused medications should be reassessed:
-
▪ alternatives should be used whenever possible;
-
▪ if still needed, they may be cautiously reintroduced after 2 months.
-
-
▪ Frequency of use should be on no more than 10 days/month:
-
▪ use on more than 6 days/month raises the risk of recidivism;
-
▪ patients should avoid treating headaches on more than 3 days in a row.
-
2.5.10 Management of trigeminal neuralgia and persistent idiopathic facial pain
-
▪ Recognition in primary care is crucial to ensure prompt referral.
-
▪ TN is extremely painful, and untreated is physically, psychologically and socially debilitating:
-
▪ patients may avoid the triggers of eating and drinking, seriously impairing food and fluid intake.
-
▪ TN therefore demands accelerated specialist referral for investigation and treatment.
-
-
▪ Good treatment begins with education of patients, explaining their disorder and the purpose and means of management.
-
▪ The objective in management, by medical or surgical means, is abatement of attacks and pain freedom. This is not always achievable.
-
▪ MRI is mandatory since classical TN and secondary TN (due usually to cerebellopontine angle tumour, AV-malformation or multiple sclerosis) may be indistinguishable by symptom presentation.
-
▪ First-line treatment is prophylactic (antiepileptic) medication.
-
▪ Acute therapies (opioids or other analgesics) have no place in management since attacks are very short-lasting.
-
▪ Severe exacerbations with anorexia and dehydration, due to pain triggered by eating or drinking, may require hospital admission for intravenous hydration and medication.
-
▪ TN produces very characteristic, very severe, electric-shock-like pains:
-
▪ along a nerve on one side of the face, usually in the cheek or jaw;
-
▪ repetitively, in short-lasting bouts (up to 2 min), which:
-
▪ occur daily for weeks or months but sometimes remit spontaneously;
-
▪ usually start without warning, but can be provoked by light touch, wind, cold air, eating, drinking, brushing the teeth or speaking.
-
-
-
▪ The cause of TN is often not known:
-
▪ some people have a blood vessel in close contact with and compressing the affected nerve: an MRI brain scan is required to show this;
-
▪ however, there are other unknown causes.
-
-
▪ Specialist referral is therefore necessary.
-
▪ There are a number of treatments for TN, which often work well:
-
▪ these are preventative medications, to be taken daily;
-
▪ painkillers do not help;
-
▪ occasionally, surgery is required, but as a last resort;
-
▪ TN does not require dental treatment.
-
First line: • Carbamazepine 200–2400 mg daily • Oxcarbazepine 600–2400 mg daily | These drugs: • reduce efficacy of oral contraceptives; • may induce hyponatraemia (especially oxcarbazepine): regular monitoring is advised; • Mmay induce osteoporosis in long-term treatment: prophylaxis against this is advised |
Second-line (either as monotherapy or as add-on medication): • Gabapentin 600–3600 mg daily • Pregabalin 150–600 mg daily • Lamotrigine 200–1000 mg daily (very slow up-titration necessary) |
-
▪ Dosages should be up-titrated slowly until pain relief is achieved or side effects become unacceptable.
-
▪ Patients established on medication may be taught to titrate up and down, according to symptom severity.
-
▪ Combinations may cause fewer side-effects because lower doses may be required of each drug.
-
▪ Treatment may be slowly tapered after complete freedom from pain, and discontinued in the absence of relapse.
-
▪ Neurosurgical treatments are relevant when medical treatment with maximum tolerated doses achieve insufficient efficacy, but:
-
▪ microvascular decompression (appropriate when neurovascular compression, not merely contact, has been demonstrated) carries a small risk of severe complications such as cranial nerve palsy or stroke;
-
▪ gamma-knife and/or percutaneous procedures (balloon compression, glycerol injection, thermocoagulation or pulsed radiofrequency treatment) targeting the trigeminal ganglion are less invasive but probably less efficacious.
-
-
▪ Patients should be educated on:
-
▪ how to taper medication cautiously once pain freedom is achieved;
-
▪ how to reintroduce medication by careful up-titration if/when pain returns.
-
-
▪ PIFP is painful, and can be physically, psychologically and socially debilitating.
-
▪ It is often difficult to manage, often has comorbidities, and usually requires specialist referral in the first instance.
-
▪ Good treatment begins with education of patients, explaining their disorder and the purpose and means of management.
-
▪ Freedom from pain is difficult to achieve: the objectives in management, by medical, physical and/or psychological therapies, are reduction of pain intensity and developing patients’ coping mechanisms.
-
▪ Treatment is prophylactic: acute therapies (opioids or other analgesics) have no place in management of PIFP.
-
▪ PIFP is most often a constant, dull, nagging or aching pain in the cheek and lower jaw. Rarely there are electric-shock-like pains also.
-
▪ There are no specific triggers.
-
▪ The causes are unknown.
-
▪ There are no tests to confirm the diagnosis.
-
▪ Preventative medications, taken every day, are the best treatments for most people with PIFP:
-
▪ these medications are more commonly used as antidepressants, but are very useful against chronic pain disorders even in people who are not depressed;
-
▪ painkillers are unhelpful and, if taken too often, are likely to make things worse.
-
-
▪ Use of drugs off-licence rests on individual clinical responsibility.
First line: • Amitriptyline or nortriptyline, 10–100 mg at night | • Intolerance is reduced by starting at a low dose (10 mg) and incrementing by 10–25 mg every 1–2 weeks; • Nortriptyline has fewer anticholinergic side-effects but less good evidence of efficacy |
Second line (either as monotherapy or as add-on medication): • Gabapentin 600–3600 mg daily • Pregabalin 150–600 mg daily |
-
▪ Patients receiving medication more often used as an antidepressant should be advised of this, and why; otherwise, they may default on finding out.
-
▪ Dosages should be up-titrated slowly until pain relief is achieved or side effects become unacceptable.
-
▪ Combinations may cause fewer side-effects because lower doses may be required of each drug.
2.6 Guides to referral
2.6.1 Headache management in primary care: when to refer
-
▪ Diagnostic uncertainty after due enquiry.
-
▪ Diagnosis of any of the following, which are best managed by specialists:
-
▪ migraine with aura including motor weakness;
-
▪ chronic migraine;
-
▪ cluster headache;
-
▪ trigeminal neuralgia;
-
▪ persistent idiopathic facial pain.
-
-
▪ Suspicion of serious secondary headache, or of serious pathology where investigation may be necessary and is not available in primary care:
-
▪ progressively worsening headache over weeks or longer;
-
▪ headache brought on by coughing, exercise or sexual activity;
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▪ headache associated with any of the following:
-
▪ postural change indicative of high or low intracranial pressure;
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▪ unexplained fever;
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▪ stiffness of the neck;
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▪ unexplained focal neurological symptoms or signs or with epileptic seizures;
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▪ disorder of consciousness or memory, or change in personality;
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▪ weight-loss or poor general condition;
-
-
▪ new headache:
-
▪ in any patient that is thunderclap in nature (intense headache with abrupt or “explosive” onset);
-
▪ that is daily and persistent from onset in a patient without a prior history of headache;
-
▪ in a patient older than 50 years;
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▪ in a patient with a history of cancer;
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▪ in a patient with a history of immunodeficiency (including HIV infection);
-
▪ in a patient with a history of polymyalgia rheumatica;
-
▪ in a patient with a family history of glaucoma;
-
-
▪ unusual migraine aura, especially:
-
▪ prolonged aura (duration >1 h);
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▪ aura featuring brainstem symptoms and/or motor weakness;
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▪ new aura without headache in a patient older than 50 years and in the absence of a prior history of migraine.
-
-
-
▪ Persistent management failure.
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▪ Comorbid disorders requiring specialist management.
3 Instruments and other materials to aid diagnosis and management of headache disorders in primary care
3.1 Introduction
-
▪ 3.3 Headache diary and calendar to aid diagnosis and follow-up in primary care) (also, Additional files 16 and 17);
-
▪ 3.4 The Headache-Attributed Lost Time (HALT) Indices: measures of burden for headache management in primary care) (also, Additional files 18 and 19);
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▪ 3.5 The Headache Under-Response to Treatment (HURT) questionnaire: a guide to follow-up in primary care) (also, Additional file 20);
3.2 Diagnostic criteria for headache disorders in primary care: the International Classification of Headache Disorders, 3rd edition (ICHD-3) – abbreviated form
3.2.1 Introduction
3.2.2 Definitions of common terms
3.2.3 Primary headaches
3.2.4 Secondary headaches
3.2.5 Painful cranial neuropathies and other facial pain
3.3 Headache diary and calendar to aid diagnosis and follow-up in primary care
3.3.1 Introduction
-
▪ symptoms and temporal patterns that contribute to correct diagnosis;
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▪ acute medication use (class, dosage and frequency), identifying base-line usage or overuse;
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▪ lost productive time as part of pre-treatment assessment.
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▪ revealing associations with the menstrual cycle and, possibly, with other triggers;
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▪ monitoring acute medication use or overuse during follow-up;
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▪ encouraging adherence to prophylactic medication;
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▪ recording treatment effect on headache frequency, and charting outcomes.
3.3.2 Diary and calendar for use in primary care
3.3.3 On-line diaries and smartphone apps
3.4 The Headache-Attributed Lost Time (HALT) Indices: measures of burden for headache management in primary care
3.4.1 Introduction
3.4.2 The Headache-Attributed Lost Time (HALT) Indices
Days lost in last 3 months | Assessed impact | Grade (indicating increasing need for medical care) |
---|---|---|
0–5 | Minimal or infrequent | I |
6–10 | Mild or infrequent | II |
11–20 | Moderate | III (indicates high need for care) |
≥20 | Severe | IV (indicates high need for care) |
3.5 The Headache Under-Response to Treatment (HURT) questionnaire: a guide to follow-up in primary care
3.5.1 Introduction
3.5.2 The Headache Under-Response to Treatment (HURT) questionnaire
4 Patient information leaflets to aid headache management in primary care (2nd edition)
4.1 Introduction
-
▪ Explanation is a crucial element of preventative management in patients with frequent migraine or tension-type headache, who are at particular risk of escalating medication consumption.
4.2 Lifting The Burden’s patient information leaflets
-
▪ accurate;
-
▪ appropriate, comprehensive, informative and helpful;
-
▪ cross-culturally relevant and understandable.
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▪ revisions (second editions) of the four leaflets on the important headache disorders in primary care:
-
▪ migraine (Additional file 21);
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▪ tension-type headache (Additional file 22);
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▪ cluster headache (Additional file 23);
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▪ medication-overuse headache (Additional file 24);
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▪ and of the fifth, explaining the relationships between female hormones and headache, which commonly raise questions from patients (Additional file 25);
-
5 Translation, and the preservation of original meaning, of materials developed to improve headache management
5.1 Introduction
5.1.1 Translation methods
5.2 Lifting The Burden’s approach, and three translation protocols
Document types | Group members | Essential criteria |
---|---|---|
Lay, such as information leaflets for people with headache; Technical, expected to be read only by professionals and used in management: management guidelines are an example; Hybrid, to be read and understood by people with headache but used either in clinical practice or in research: examples are lay-administered diagnostic questionnaires, diagnostic or follow-up diaries, the HALT Indices (measures of impact) and the HURT questionnaire (an outcome measure). | JM Bertolote, Department of Mental Health and Substance Abuse, World Health Organization, Geneva, Switzerland; C Houchin, Oxford Outcomes Ltd., Oxford, UK; T Kandoura, Oriental Institute, University of Oxford, Oxford, UK; M Peters, Nuffield Department of Population Health, University of Oxford, Oxford, UK TJ Steiner, Division of Brain Sciences, Imperial College London, London, UK | The protocols must: • Conform to accepted translation guidelines; • Ensure rigour of the translation process and quality of the translated products; • Be suitable and have utility across different countries and cultures; • Include target-user evaluation; • Be pragmatic, recognising that unduly onerous protocols would be rejected and therefore unhelpful. |
5.2.1 Translation protocol for lay documents (2nd edition)
-
▪ bilingual in English and the target language (ideally a native speaker and resident of the country of the target language);
-
▪ has ability to mediate between different translators and to understand the points of view of lay and professional translators.
-
▪ selecting the translators, assessor and review panel (and referee when necessary);
-
▪ organising and overseeing the translation, including meeting with the translators to produce a consensus-based translation;
-
▪ organising and overseeing the quality assurance of the translation;
-
▪ producing the report of the translation process.
-
▪ native speaker of the target language;
-
▪ at least one (individual, pair or panel) must be headache or medical expert(s)
-
▪ (ideally, the other is a professional translator or bilingual person, pair or panel skilled in language/linguistics, such as a teacher or journalist; if no such translator is available, then a second headache or medical expert [individual, pair or panel] may be used).
-
▪ keep translations simple, avoiding technical language, so that the documents can be understood by lay people of average reading ability;
-
▪ make semantic and conceptual translations (rather than literal), so that the meanings of the words and phrases remain as in the original document;
-
▪ keep a record of any parts that they found difficult to translate.
-
▪ the translators each send their translations to the coordinator;
-
▪ the coordinator makes an initial comparison of the two translations and highlights and records any parts of them that are substantially different;
-
▪ the coordinator and translators (or leads) meet (or, alternatively, hold a teleconference) to discuss these parts and any other problem areas, agreeing through consensus on one translation.
-
▪ a lay person (not medically qualified and not a researcher);
-
▪ a native speaker of the target language (and, ideally, a resident of the relevant country) with good understanding of linguistic factors (such as grammar, readability) but not necessarily bilingual.
-
▪ that the document is to be understood by lay people of average reading ability;
-
▪ to assess the consensus-based translation for readability, grammatical correctness and cultural suitability;
-
▪ to keep a record of his/her comments and send these to the coordinator.
-
▪ affected by headache disorders;
-
▪ native speakers of the target language and not necessarily bilingual.
-
▪ the original document;
-
▪ the two first translations, the consensus-based translation, any other intermediate versions and the final translation;
-
▪ a record of any substantial difficulties encountered during the translation (difficulties may include problematic words or parts of the document that were difficult to translate, points of disagreement and alternatives, or any aspects on which it was difficult to achieve consensus or that were highlighted during the quality assessment of the translation).
5.2.2 Translation protocol for technical documents (2nd edition)
-
▪ a headache expert;
-
▪ bilingual in English and the target language (ideally a native speaker and a resident of the country of the target language);
-
▪ has ability to mediate between different translators and to understand the points of view of lay and professional translators.
-
▪ selecting the translators and assessors (and referee when necessary);
-
▪ organising and overseeing the translation, including meeting with the translators to produce a consensus-based translation;
-
▪ organising and overseeing the quality assurance of the translation;
-
▪ producing the report of the translation process.
-
▪ native speakers of the target language;
-
▪ at least one (individual, pair or panel) must be headache expert(s) or primary-care physician(s), according to the intended audience of the document;
-
▪ (ideally, the other is a professional translator or bilingual person, pair or panel skilled in language/linguistics, such as a teacher or journalist; if no such translator is available, then a second headache expert or primary-care physician [individual, pair or panel] may be used).
-
▪ keep translations professional, using technical language;
-
▪ make semantic and conceptual translations (rather than literal), so that the meanings of the words and phrases remain as in the original document;
-
▪ avoid invention (adding their own ideas to the text);
-
▪ keep a record of any parts that they found difficult to translate.
-
▪ the translators each send their translations to the coordinator;
-
▪ the coordinator makes an initial comparison of the two translations and highlights and records any parts of them that are substantially different;
-
▪ the coordinator and translators (or leads) meet (or, alternatively, hold a teleconference) to discuss these parts and any other problem areas, agreeing through consensus on one translation.
-
▪ either headache experts or primary-care physicians, according to the intended audience of the document;
-
▪ native speakers of the target language (and, ideally, a resident of the relevant country) with good understanding of linguistic factors (such as grammar, readability) but not necessarily bilingual.
-
▪ that the document is to be utilized by health-care professionals (specified, when appropriate);
-
▪ to assess the consensus-based translation for readability, grammatical correctness, medical correctness and cultural suitability;
-
▪ to keep records of their comments and send these to the coordinator.
-
▪ the original document;
-
▪ the two first translations, the consensus-based translation, any other intermediate versions and the final translation;
-
▪ a record of any substantial difficulties encountered during the translation (difficulties may include problematic words or parts of the document that were difficult to translate, points of disagreement and alternatives, or any aspects on which it was difficult to achieve consensus or that were highlighted during the quality assessment of the translation).
5.2.3 Translation protocol for hybrid documents (2nd edition)
-
▪ has technical knowledge (ie, understands the concepts underlying the questions or instrument being translated);
-
▪ bilingual in English and the target language (ideally a native speaker and a resident of the country of the target language);
-
▪ has ability to mediate between different translators and to understand the points of view of lay and professional translators.
-
▪ selecting the forward- and back-translators, assessor and review panel (and referee when necessary);
-
▪ liaising when necessary with the document author;
-
▪ organising and overseeing the forward- and back-translations, including meeting with the translators first to produce a consensus-based forward-translation and again (when necessary) to resolve discrepancies discovered during back-translation;
-
▪ organising and overseeing the quality assurance of the translation;
-
▪ producing the report of the translation process.
-
▪ native speaker of the target language;
-
▪ at least one (individual, pair or panel) must be headache or medical expert(s);
-
▪ (ideally, the other is a professional translator or bilingual person, pair or panel skilled in language/linguistics, such as a teacher or journalist; if no such translator is available, then a second headache or medical expert [individual, pair or panel] may be used).
-
▪ keep translations simple, avoiding technical language, so that the documents can be understood by lay people of average reading ability;
-
▪ make semantic and conceptual translations (rather than literal), so that the meanings of the words and phrases remain as in the original document;
-
▪ keep a record of any parts that they found difficult to translate.
-
▪ the translators each send their translations to the coordinator;
-
▪ the coordinator makes an initial comparison of the two translations and highlights and records any parts of them that are substantially different;
-
▪ the coordinator and translators (or leads) meet (or, alternatively, hold a teleconference) to discuss these parts and any other problem areas, agreeing through consensus on one forward translation.
-
▪ a native speaker of English;
-
▪ either a headache or medical expert, or a professional or bilingual lay translator skilled in language/linguistic issues.
-
▪ a lay person (not medically qualified and not a researcher);
-
▪ a native speaker of the target language (and, ideally, a resident of the relevant country) with good understanding of linguistic factors (such as grammar, readability) but not necessarily bilingual.
-
▪ that the document is to be understood by lay people of average reading ability;
-
▪ to assess the back-checked consensus-based translation for readability, grammatical correctness and cultural suitability;
-
▪ to keep a record of his/her comments and send these to the coordinator.
-
▪ affected by headache disorders;
-
▪ native speakers of the target language and not necessarily bilingual.
-
▪ the original document;
-
▪ the two forward-translations, the consensus-based translation, the back-translation, the back-checked consensus-based translation, any other intermediate versions and the final translation;
-
▪ a record of any substantial difficulties encountered during the translation (difficulties may include problematic words or parts of the document that were difficult to translate, points of disagreement and alternatives, or any aspects on which it was difficult to achieve consensus or that were highlighted during the quality assessment of the translation).